A patient underwent four ultrasounds during her pregnancy. The sonographer remarked that in three of the studies, there were, “Structural irregularities that require further evaluation.” The physician notified the patient that the ultrasounds were normal. The baby was born with a midline facial defect, cleft palate, club foot, and lower-limb anomalies. The child has limited cognitive and communication skills. During litigation, the physician admitted that he had not reviewed the images from the studies or the sonographer’s handwritten report about the findings.


	A $1.9 million settlement was reached prior to trial.


	Unfortunately, it is not uncommon for physicians to rely on the sonographer’s “report” and fail to personally review the images. Although sonographers are responsible for obtaining appropriate images, interpreting ultrasounds is beyond the scope of their professional practice. Interpretation of studies and associated recommendations are the responsibility of the physician. Physicians should review study images and either scan the patient themselves or refer the patient to an imaging specialist for further evaluation and diagnosis.

Documenting and reading the report carefully


	A 28-year-old G3P2002 presented to her physician at 16 weeks, 4 days with a history of oligomenorrhea and two prior cesarean deliveries. Her estimated date of delivery (EDD) was 4/10/XX. Because of the woman’s body habitus, the physician was unable to palpate the uterine fundus. Fetal heart tones were documented at 160 beats per minute. The patient was referred for an ultrasound, which was performed at 17 weeks’ gestation by dates and consistent with 9 weeks, 4 days’ gestation. The report stated, “Live, intrauterine pregnancy with a gestational age of 9 weeks 4 days + 6 days. The EDD is 4/10/XX. No abnormalities visualized.” The EDD should have been 6/2/XX but the report showed the original EDD, rather than the new one. No further ultrasounds were performed during the pregnancy. 

On 4/05/XX, the patient delivered a 1710-g male infant via cesarean, who had Apgar scores of 9 and 9 at 1 and 5 minutes, respectively. The baby’s 

 was consistent with 31 weeks. He suffered unusually severe complications of prematurity with severe respiratory distress syndrome, bronchopulmonary dysplasia, and necrotizing enterocolitis requiring surgery. 

Deposition of the defendant obstetrician revealed repeated exam inconsistencies and poor documentation. For example, the patient was seen for abdominal pain at 23 and 2/7 weeks by dates and 15 and 5/7 weeks by ultrasound. The only documentation of the examination was “Uterus is normal.” There were repeated discrepancies between the fundal height and the estimated gestational age. The plaintiff’s expert witness testified that the ultrasound report erroneously reported the estimated date of delivery, the primary obstetrician should have recognized this discrepancy and the persistent discrepancy in the fundal height and gestational age and ordered a repeat ultrasound. 


	The case settled for $980,000 prior to trial. 


	Imaging specialists must construct their report to alert referring physicians to significant sonographic findings or notify the referring physician of them personally or through their appropriate delegate, such as that physician’s nurse. Recommendations for further studies are integral to a complete report. For example, in the referenced case, a preferred report follows:

The estimated gestational age by ultrasound is 9w4d. This is not consistent with the estimated age by dates. 

 recommends adjusting the EDD if the discrepancy is more than 7 days when the gestational age is between 9w0d and 13w6d. Thus, 

the EGA should be adjusted to 9w4d, with an EDD = 6/03/XX.

 The adjusted EDD should be confirmed on subsequent ultrasound studies. Consider a nuchal translucency at 11-14 weeks. An anatomic survey is recommended at 18-20 weeks EGA. 

The final written report is considered the definitive means of communicating the results of an imaging study or procedure. Direct or personal communication must occur in certain circumstances, such as major fetal anomalies or findings that immediately impact management of the pregnancy. The primary obstetrician must read the entire report and correlate the ultrasound findings with the clinical findings. Inconsistencies require further investigation or imaging. 

Be careful about what you DO NOT document.


	A 33 year-old G3P2002 underwent an ultrasound at 19 and 1/7 weeks’ gestation. The ultrasound reported stated, “Normal ultrasound with fetus at 19 1/7 weeks of gestation.” No further ultrasounds were performed. At 39 weeks’ gestation, the patient delivered a baby with Down syndrome. An expert review of the ultrasound revealed mild pyelectasis with calyceal dilatation of 4.3 and 4.4 mm. In addition, an echogenic intracardiac focus was identified. At trial, the radiologist testified that the practice rounds to the nearest whole number. Thus, the calyceal dilation would have been 4 mm and within normal limits. Further, an echogenic intracardiac focus is a worthless marker and of no consequence. Thus, the ultrasound was normal. 

It is the obstetrician’s duty to recommend further testing to the patient. The obstetrician testified that the ultrasound was reported as normal and he had no reason to recommend amniocentesis or further ultrasound studies. The plaintiff’s expert testified that calyceal dilatation > 4 mm at 19 and 1/7 weeks’ gestation warrants a repeat ultrasound at 32 weeks to evaluate for persistence of the calyceal dilatation. As an isolated finding, an echogenic focus is poor marker for Down syndrome. However, when multiple soft markers for Down syndrome are identified, they should be noted in the report and recommendations made to recalculate the patient’s risk with amniocentesis, if indicated. A repeat ultrasound should have been recommended.


	The jury found as follows:
	Obstetrician: Defense verdict
	Radiologist: Plaintiff verdict. The radiologist had a duty to report the findings to the obstetrician. If he had done so, the duty for further counseling, evaluation, and treatment would have transferred to the obstetrician. 


	Imaging specialists, regardless of their specialty, should report all visualized abnormal findings, recommending clinical correlation and further studies as indicated. Significant or concerning findings may require direct communication with the referring physician or his or her delegate. Further, this case demonstrated a rather common error made during trial: One defendant criticizes the care or blames the outcome on another defendant. Such actions render a case virtually indefensible. Defendants should not act as experts for the plaintiff. At trial, a defendant should confine his or her testimony to their actions and rationale, while avoiding criticizing the care of others. 

Be careful what you bill and how you document

A radiology group routinely performs and bills for transabdominal and transvaginal ultrasound (TVUS) studies in all patients referred for a pelvic ultrasound. They came under scrutiny for billing fraud, for inappropriately billing for both studies in all patients, particularly as they could not identify an order requesting both studies from referring physicians.


	The Department of Justice fined the group $10 million for performing “unnecessary procedures.”


	Many ultrasonographers opine that the standard of care requires the performance of both an abdominal and a vaginal sonogram at the time of pelvic ultrasound. This is not the standard of care. Certainly, if all relevant anatomy cannot be identified with one approach, appropriate evaluation requires the additional approach. Whether a specific order is required is controversial. If a practice performs its own ultrasound studies, this is a minor issue, as the providers are readily available to confirm the need for additional study. However, imaging specialists who see many referred patients face greater obstacles, particularly in the era of electronic health records and electronic orders. 

Several recommendations can be made for ordering, reporting, and billing. (See Sidebar: Options for overcoming the issue of performing studies without appropriate orders)

Incorporating language like what is shown in the sidebar into the electronic reporting system can facilitate its inclusion in an ultrasound report. 

Billing for both studies must be appropriate. For example, if an ovary not seen on TVUS could be visualized on abdominal ultrasound, it would be appropriate to bill CPT 76830 (Complete evaluation of the female pelvic anatomy-vaginal study) and CPT 76857 (Ultrasound, pelvic [non-obstetric], real-time with image documentation; limited or follow-up).

 Billing for CPT 76856 (Complete evaluation of the female pelvic anatomy-abdominal study) should not be done unless all elements of the abdominal ultrasound are performed. 

Description and measurements of uterus and adnexal structures

Measurement of the bladder (when applicable)

In general, the code for a limited study, whether transabdominal or transvaginal, is the more common second code. It documents that the study did not include all elements of a complete study. 

Inaccurate or inappropriate billing may present greater financial risk than medical liability. The physician is ultimately responsible for submitting the correct bill. Delegating coding for procedures increases the risk of error, for which the provider is liable. Electronic health records highlight another risk of possible fraudulent billing. Including the documentation for a management consult in the ultrasound report is not adequate for the purpose of billing an evaluation and management (E&M) code. Documentation in a separate progress note should be performed when billing an E&M code, in addition to the CPT code(s) for an ultrasound study.

These cases illustrate common errors leading to litigation when performing obstetric and gynecologic ultrasound. Underlying each scenario is proper documentation of the ultrasound findings. Findings should be recorded and results communicated consistent with the AIUM Practice Parameter for Documentation of an Ultrasound Examination.

 Applying the discussed recommendations is no guarantee that a physician will not be held liable for such errors. Applying these best practices, however, will enhance the defensibility of such cases.

AMA AMA. CPT/Professional. In: AMA, ed. Chicago, IL: AMA; 2019:495-497.

AIUM. AIUM Practice Parameter for Documentation of an Ultrasound Examination.  Practice Parameter. Laurel, MD: AIUM; 2014.

Articles

Four cases illustrate common errors that can lead to litigation when performing obstetric and gynecologic ultrasound.

Ultrasound errors to avoid: How important is the report?

 from the US market has created concern among patients with the device about the reason for the manufacturer’s action. Many women also may have questions about whether to have the device removed. This article reviews the history, literature, and symptoms related to Essure as well as removal techniques to guide physicians through the counseling process. 

Essure is a hysteroscopic sterilization technique involving placement of nickel-titanium microinserts in the fallopian tubes. Over the course of
	12 weeks, the microinserts cause fibrosis and occlusion of the fallopian tubes. Essure was approved by the US Food and Drug Administration (

 Benefits of the technique were that it was a minimally invasive procedure that could be conveniently performed in the office without requiring general anesthesia or abdominal incisions and hormone-free sterilization. It was a safe option for women who had contraindications to general anesthesia or hormonal contraception or who wanted to avoid abdominal surgery.

Increasing patient reports about Essure-related symptoms led to a growing number of safety concerns about the device. Patient concerns have ranged widely from hair loss to physical and mental disabilities.

 One retrospective cohort study found that the most commonly reported symptoms following Essure placement were abdominal pain, back pain, fatigue, leg and hip pain, dysmenorrhea, and heavy menstrual bleeding.

 Another retrospective case series revealed that pelvic pain, abnormal uterine bleeding (

), and a reported allergic reaction were the symptoms most commonly reported by women prior to undergoing Essure removal.

 Patients who reported allergic reactions prior to sterilization were at higher risk of developing an allergic reaction with hysteroscopic sterilization when compared to laparoscopic sterilization.

Laparoscopic Essure Tubal Reversal: How We Do It

Websites and Facebook groups run by women with Essure who had symptoms, led to a public outcry against the device, specifically in regards to the polyethylene terephthalate (PET) fibers in the microinserts.

 In response, in 2015, the FDA appointed a special panel to investigate these claims, added a “black box” warning, and created stricter guidelines for placement.

 Continued complaints and stricter guidelines caused a significant decrease in the number of devices placed.

 Bayer removed Essure from the European market in September 2017

 and from the US market in December 2018 due to the decrease in sales.

 In its announcement, Bayer stated that preventative removal for those without symptoms was unnecessary.

Sales of controversial contraceptive implant to end

Gynecologists may see patients who have multiple concerns about the Essure device, including a variety of symptoms and questions raised by recent media coverage. When a woman presents with an Essure-related complaint, it’s important to take a detailed history. Patients may report multiple symptoms that may or may not be associated with the device and each should be documented and assessed. For each symptom, we recommend determining whether it is localized or generalized and identifying the timing of onset, severity, aggravators, or relievers. Patients may experience symptom onset from immediately to several years after Essure placement. We recommend documenting treatment attempts, including non-pharmacologic and pharmacologic treatments. In addition, because patients may also seek care from other consulting providers-such as allergists, rheumatologists, gastroenterologists or neurologists-it may be beneficial to collaborate with these consultants to determine a care plan.

For symptomatic patients, we recommend a detailed physical exam with a pelvic exam. It is particularly important to use a systematic approach to an abdominopelvic exam in cases of pain-related symptoms prior to surgery to eliminate other causes of pelvic pain. This type of exam includes an abdominal exam and may start with assessment of sensation with light touch or a pinprick. Next, lightly palpate to help identify any trigger points related to myofascial pelvic pain syndrome. Next, deep palpation can assess for any masses or hernias. Finally, assess for 

, which if positive, suggests an abdominal wall etiology. Pelvic exam should first include a Q-tip test along the external genitalia to evaluate for vulvodynia and assess sensation. Next, proceed with internal palpation of the obturator internus and levator ani to evaluate for pelvic floor myofascial pain. A bimanual exam can assess uterine size and adnexa. Finally, perform a speculum exam to observe for anatomic abnormalities or evaluate for infectious etiologies.

Additional laboratory studies may be helpful in some patients. We recommend a work-up for AUB, which may include a complete blood count, thyroid screening, Pap test, and assessment of the endometrium with an endometrial biopsy. Transvaginal ultrasound (US) is helpful for diagnosis and surgical planning to evaluate uterine size, uterine structural abnormalities, or any adnexal masses. 

In asymptomatic patients who present with concerns about safety of Essure following removal from the market, conversation and detailed education is necessary. A pelvic exam may or may not be indicated. 

A range of imaging techniques can be used to evaluate microinsert placement, including ultrasonography, hysterosalpingography (HSG), and abdominal pelvic x-ray. Imaging can confirm placement of the device and may inform counseling regarding device removal or surgical technique. We recommend ultrasonography, as it may elucidate other causes of symptoms such as fibroids, endometrial polyps, or ovarian cysts such as endometriomas. In addition, ultrasonography may also show perforation of the device through the uterus or fallopian tubes. HSG and x-ray imaging can confirm tubal occlusion and evaluate the implants, with the possibility of revealing fractured implants.

Several techniques for removal of Essure have been documented, including use of hysteroscopy, laparoscopic salpingectomy, cornuectomy, and total hysterectomy.

 The type of removal chosen may depend on a patient’s symptoms and comorbidities, and the surgeon’s skill and experience. When discussing surgery with a patient, it is important to elicit her concerns, including preference for an approach, recovery time, and desire for en bloc removal. Because most patients with Essure want permanent sterilization, we do not discuss fertility-preserving techniques here. 

Hysteroscopic Essure removal can be an option up to 6 weeks after placement, before tubal occlusion has occurred.

 This method of removal can be performed in situations of acute post-procedural pain and may help avoid abdominal surgery. Hysteroscopic removal also can be performed in conjunction with a laparoscopic salpingectomy to ensure removal of all filaments.  

Laparoscopic bilateral salpingectomy has been well-documented as a removal method; however, there is a theoretical risk of incomplete removal.

 This approach can often be performed with three 5-mm laparoscopic ports. The surgeon makes an incision in the fallopian tube with electrocautery approximately 1 cm from the cornua. The Essure device is then grasped and gentle traction is used to remove both the outer and inner coils from the cornua. Next, electrocautery is used along the mesosalpinx to remove the fallopian tube.

 Postoperative recovery time is usually 2 to 4 weeks. Benefits of this approach include the ability to diagnose and remove any pelvic pathology, that could also be contributing to the patient’s symptoms. This technique, however, has the potential to leave fragments of the microinsert device, PET fibers, in the pelvis or in the uterus as the microinsert is pulled from the cornua.  In addition, we counsel patients that they may require another surgery if their symptoms continue postoperatively. 

Laparoscopic cornuectomy with bilateral salpingectomy, an approach used to remove the microinsert and fallopian tubes en bloc, requires a laparoscopic surgeon trained in cornual wedge resection. It may require a 10-mm and two 5-mm laparoscopic ports, vasopressin for hemostasis, and suturing of the cornua in multiple layers. Although this technique has increased risks, including blood loss,

 patients may benefit from complete removal of the device. It may also be helpful in patients with symptoms concerning for allergic reaction. Patients may require 4 to 6 weeks for recovery. 

Hysterectomy with bilateral salpingectomy will also remove the Essure inserts and can be done vaginally, laparoscopically or abdominally. This technique may be desirable for patients who also have symptoms of AUB and/or adenomyosis. Hysterectomy has the greatest surgical risk of all Essure removal techniques, including infection, hemorrhage, and ureteral injury. Recovery time for this method is the longest, at 6 to 8 weeks.

 In one study of women who underwent gynecologic surgery following Essure placement, hysterectomy was the most commonly performed surgery.

 This may be because it is the most likely to treat multiple symptoms, including Essure-related allergy, pelvic pain, and AUB. 

Several case studies and prospective and retrospective research have looked at symptom relief after Essure removal. Studies have reported that 40% of patients have complete resolution of symptoms when followed up to 3 years after removal via hysteroscopic, laparoscopic and laparotomy removal.

  This unfortunately means that most patients do not have complete resolution of symptoms after removal. In addition, 10% to 15% reported no change in symptoms, with abdominopelvic pain being the most commonly experienced symptom prior to and following the surgeries previously mentioned.

 Another study showed 75% improvement in quality-of-life following surgery, which was similar for patients undergoing laparoscopic bilateral salpingectomy or hysterectomy for removal.

 A number of case series have proven that patients with previous nickel allergy or who develop allergy after Essure placement have improved outcomes after laparoscopic or hysteroscopic removal.

 In a follow-up study of medical outcomes after sterilization, there was no difference in risk of developing an autoimmune disease with hysteroscopic versus laparoscopic sterilization.

One difficulty in assessing surgical success is that it may also be hard to distinguish between resolution due to removal due to placebo effect. 

Patients with the Essure device may present with or without symptoms related to the microinsert. Women without symptoms should be counseled that they do not need to have their coils removed and that removal could cause increased risk of injury. Symptomatic patients who want counseling about Essure removal should be worked up for the most common differential diagnoses for their symptoms. A thorough history and physical exam is crucial to identifying possible causes. Specific labs, imaging, and consultation with multidisciplinary consultants may also assist with diagnoses unrelated to Essure. Counseling regarding methods of removal and outcomes associated with them are necessary for shared patient decision-making to achieve a patient’s desired outcomes. 

The authors report no potential conflicts of interest with regard to this article.

Bayer. Frequently Asked Questions About Essure.

Banks C, et al. Increasing patient engagement in pharmocovigilance through online community outreach and mobile reporting applications: an analysis of adverse event reporting for the Essure Device in the US. Pharm Med. 2015;29(6):331-340.

Maassen LW, van Gastel DM, Haveman I, Bongers MY, Veersema S. Removal of Essure sterilization devices: a retrospective cohort study in the Netherlands. J Minim Invasive Gynecol. 2018 Oct 16. pii: S1553-4650(18)31302-5.

Clark NV, Rademaker D, Mushinski AA, Ajao MO, Cohen SL, Einarsson JI. Essure removal for the treatment of device-attributed symptoms: an expanded case series and follow-up survey. J Minim Invasive Gynecol. 2017;24(6):971-976.

Bouillon K, Bertrand M, Bader G, Lucot JP, Dray-Spira R, Zureik M. Association of hysteroscopic vs laparoscopic sterilization with procedural, gynecological, and medical outcomes. JAMA. 2018;319(4):375-387.

Advocating Safety in Healthcare E-Sisters (ASHES). 

Bayer. New Release: Bayer to voluntarily discontinue U.S. sales of Essure at end of 2018 for business reasons. h

ttp://www.essure.com/assets/pdf/PP-250-US-1923-FINAL-News-Release.pdf

Meister MR, Sutcliffe S, Ghetti C, et al. Development of a standardized, reproducible screening examination for assessment of pelvic floor myofascial pain. Am J Obstet Gynecol. 2019;220(3):255.e1-255.e9.

Sills ES, Dalton MM. Referrals for complications following hysteroscopic sterilisation: characteristics associated with symptomatic patients after the Essure procedure. Eur J Contracept Reprod Health Care. 2016;21(3):227-233.

Charavil A, Agostini A, Rambeaud C, Schmitt A, Tourette C, Crochet P. Vaginal hysterectomy with salpingectomy for Essure insert removal. J Minim Invasive Gynecol. 2019;26(4):695-701.

Sills ES, Li X, Wood SH, Jones CA. Analysis of surgeries performed after hysteroscopic sterilization as tabulated from 3,803 Essure patient experiences. Obstet Gynecol Sci. 2017;60(3):296-302.

Thiel L, Rattray D, Thiel J. Laparoscopic cornuectomy as a technique for removal of Essure microinserts. J Minim Invasive Gynecol. 2017;24(1):10.

Albright CM, Frishman GN, Bhagavath B. Surgical aspects of removal of Essure microinsert. Contraception. 2013;88(3):334-336.

Lazorwitz A, Tocce K. A case series of removal of nickel-titanium sterilization microinserts from the uterine cornua using laparoscopic electrocautery for salpingectomy. Contraception. 2017;96(2):96-98.

Lane A, Tyson A, Thurston E. Providing Re-Essure-ance to the nickel-allergic patient considering hysteroscopic sterilization. J Minim Invasive Gynecol. 2016;23(1):126-129.

When a patient is interested in having the device removed, her physician needs to examine several factors before deciding on a surgical approach.

Considerations when discussing Essure removal

Just like our patients, proper coding and billing for obstetric patients can be…complicated. As a coding instructor and compliance auditor, I field a lot of questions from new students and experienced billers alike. In this article, I’ll break down a few of the most important concepts you need to understand to master obstetric coding.
	 

Billing codes for maternity care and delivery are used to report antepartum care, vaginal or cesarean delivery, and postpartum care in uncomplicated pregnancies. Antepartum care includes the initial prenatal history and examination, subsequent prenatal history and examinations, recording of weight, blood pressures, fetal heart tones, routine chemical urinalysis, and monthly visits up to 28 weeks’ gestation; biweekly visits to 36 weeks’ gestation; and weekly visits until delivery. Delivery services include the hospital admission, management of uncomplicated labor, and cesarean or vaginal delivery (including episiotomy and repair if needed). Postpartum care includes visits in the hospital and a 6-week follow-up in the office following delivery. It may also include services related to a cesarean delivery, such as an incision check.  

When billing for maternity care and delivery, codes exist to bill the global package (antepartum care, delivery, and postpartum care) or for specific components in the event you are billing for a provider who performed the antepartum care only, delivery only, or postpartum care only.  

Note that pregnancy confirmation during a problem-oriented or preventive visit is not considered part of antepartum care and should be reported separately using the appropriate E/M code (for example an 

Outpatient Office Visit Code, 99201-99215

). Also, remember that medical complications of pregnancy (eg, cardiac problems, neurological problems, diabetes, hypertension) and medical problems complicating labor and delivery management may require additional resources and should be billed separately – they are not included in the “global OB package.”

When it comes to billing for maternity care and delivery, the number of code options can be confusing. Simply put, there are codes to report the global package, or components of it (as discussed above) depending on the method of delivery. For example, 

 is used to report Routine OB care including antepartum care, 

, and postpartum care. Codes immediately following 59400 report individual components of the global package. Code 

 reports Routine OB care including antepartum care, 

However, there’s one more wrinkle that confuses some coders: Patients who have had a previous cesarean delivery and now present with an expectation of vaginal delivery have their own set of delivery codes, depending on the outcome. 

 reports routine OB care including antepartum care, vaginal delivery, and postpartum care 

 is used when the same patient with a previous cesarean attempts a vaginal delivery but 

With ICD-10 OB coding came a new set of billing guidelines that can be complicated, especially for newer coders trying to get acclimated to coding. The first important consideration when selecting a diagnosis code is to read the guidelines for each section. The ICD-10 guidelines state that codes for chapter 15 (

Complications of Pregnancy, Childbirth, and the Puerperium

) have sequencing priority over other codes from other chapters and are to be listed first unless the pregnancy is completely incidental and has nothing to do with the reason for the visit.  

Another important consideration is the fact that in ICD-10, most of the diagnoses in chapter 15 include the concept of trimester. The guidelines state that not only should you select the code with the appropriate trimester for the current encounter,  but a separate code (Z3A.--) should be used to report the weeks gestation for the encounter. For example, a patient in her 16

 week of pregnancy should be assigned a code from chapter 15, followed by the code Z3A.16 to indicate 16 weeks’ gestation. 
	 

For a routine outpatient prenatal visit when no complications are present, select a diagnosis code from range Z34, Encounter for Supervision of Normal Pregnancy, as the primary diagnosis. Remember that on average, the global OB package encompasses 13 routine visits during pregnancy, which includes routine visits in uncomplicated cases, and 6 weeks postpartum care. This may include H&Ps, routine measurements, and educational services such as breastfeeding or basic newborn care. Services that are not part of the global OB package may be billed separately, such as an initial evaluation and management service to diagnose pregnancy, amniocentesis, cephalic version, additional E&M services for unrelated reasons (e.g., asthma or urinary tract infection) or greater frequency of visits due to a high-risk pregnancy.  Sometimes coders get confused and either miss out on billing a service that isn’t included in the global OB package or they mistakenly try to bill for a visit that is included in the package.  

Sometimes our patients (and our coding) can be routine, but other times things can get complicated.  Having a good understanding of what is included can help you be prepared so that when you encounter a situation that isn’t included, you don’t forget to bill for it. It always helps to have your (

) coding manuals and/or cheat sheets nearby, and reimbursement policies from your office’s biggest payers on your computer.

The author reports no potential conflicts of interest with regard to this article.

The sheer number of code options can be intimidating, but mastering a few concepts can alleviate some of the stress.

Is it possible to make OB coding less complicated?

) is a devastating blood disorder with particular implications for women. In the 1970s, the life expectancy for a person with SCD in the United States was approximately 10 years.

 Now, the life expectancy is approximately 50 years.

 Girls born with SCD in this century can expect to live to and through their childbearing years. Their blood disorder, however, presents unique challenges at every life stage that ob/gyns need to know how to manage.  

SCD is an autosomal-recessive disease characterized by presence of sickled red blood cells (RBCs). Sickled RBCs form in an individual who is homozygous for the sickle hemoglobin (HbS) gene (SS genotype) or is heterozygous for HbS and has another abnormal hemoglobin such as hemoglobin C (SC genotype), beta thalassemia (S-beta thal+ or S-beta thal

 genotype), or some other rare hemoglobin. HbS has a single amino acid substitution of valine for a glutamic acid in the beta chain of the hemoglobin molecule, which prevents hemoglobin from forming neat tetramers. Instead the hemoglobin forms long, fibrous polymers that distort RBC membranes. These distorted RBCs are readily destroyed by the reticuloendothelial system. The normal life span of sickled RBCs is approximately 15 days compared to the 120 days of normal RBCs.

 Consequently, individuals with SCD suffer from moderate to severe anemia. Table 1 lists the prevalence of various genotypes derived from California newborn screening data

 and the severity of the various genotypes.

 The SS genotype (which accounts for more than half of the affected individuals in the United States) and the S-beta thal

 genotype generally result in a more severe phenotype described as “sickle cell anemia.” The other genotypes do not usually result in severe disease.  

Anemia is not the only mechanism of the disease. Lysed RBCs release free hemoglobin, which consumes nitric oxide and leads to endothelial damage and possibly thrombosis. Membrane receptors become rearranged on the distorted RBC surface, altering its adhesive properties. These altered RBCs interact with the endothelium, white blood cells, and platelets, and contribute to intravascular congestion, thrombosis, and downstream ischemia, resulting in both acute and chronic tissue damage.  

SCD affects approximately 1 million individuals worldwide, two-thirds of whom live in West Africa. Unfortunately, mortality for children with SCD in low- and middle-income countries is 50% to 90%. In the United States, approximately 100,000 Americans are affected with SCD, mostly individuals of African descent, but also Mediterranean, Middle Eastern, and Indian descent.

 Forty years ago, 50% of children in the United States born with SCD also died before adulthood and 30% died before their fifth birthday,

 but since the 1970s, newborn screening programs, institution of penicillin prophylaxis, vaccinations against encapsulated organisms, and disease-modifying therapy with hydroxyurea have led to  a dramatic decline in childhood mortality. SCD is no longer a life-threatening disease of childhood, but now a chronic disease of adults.

SCD affects almost every organ and organ system, as shown in Table 2. Acute complications of the disease are listed in Table 3.

 The cost of care, estimated from a study of Florida Medicaid claims from 2001-2005, averaged $1389 per month per patient.

Of particular concern to ob/gyns are the maternal and fetal consequences of SCD. Pregnancy complications are summarized in Table 4. During normal pregnancy, there is a demand for increased erythropoiesis. Because women with SCD are already anemic, 30% to 40% require transfusion during pregnancy.

 During normal pregnancy, women have an increased susceptibility to certain infections. The risk of infection is compounded in women with SCD. Compared to women without SCD, infectious morbidity is increased 2- to 13-fold. During normal pregnancy, women have an increase in glomerular filtration. Pregnancy has the potential to further impair renal function in women with sickle cell nephropathy. Normal pregnancy results in an increase in cardiac output. In a woman with SCD, the increased cardiopulmonary demands of pregnancy are potentially life-threatening, especially in women with SCD-induced pulmonary hypertension. Pregnancy results in an increased risk of thrombosis. In women with SCD who are already at high risk of VTE and stroke, pregnancy increases the risk of thrombosis 2- to 5-fold compared to women without the disease. Preeclampsia is increased 6- to 8-fold in women with SCD and maternal mortality is increased 6-fold, compared to women without SCD.

Fetal growth appears to start out normally, then lags after 25 weeks’ gestation.

 Fetal consequences of SCD include a 2-fold increased risk of preterm birth,

 a 3-fold risk of small-for-gestational age,

 and a 4-fold increased risk of stillbirth.

Diagnosis of SCD within the first 3 months of life allows for early treatment. Since 2006, every state in the United States has had a newborn screening program for the disease. A study conducted in California found that overall mortality for children who were diagnosed after they presented with symptoms was 8%, compared to 1.8% after early identification of SCD through screening and accompanying education of providers.

Treatment for SCD consists of preventing complications, managing pain, modifying the disease, or attempting a cure. Prevention of complications includes prophylactic penicillin for children to prevent sepsis and meningitis from encapsulated bacteria, and vaccinations against 

 Individuals with SCD are particularly vulnerable to these encapsulated bacteria due to functional or surgical asplenia. Children with SCD are also particularly vulnerable to stroke. Children with abnormal transcranial Doppler velocities are prescribed long-term transfusion therapy, which has been shown to dramatically reduce incidence of stroke.

Managing the acute and chronic pain of SCD is challenging. The mainstay of pain management has been opioids, but nonsteroidal anti-inflammatory drugs (NSAIDs) have also been used. Particularly for management of chronic pain, amitriptyline, gabapentin, selective serotonin reuptake inhibitors (SSRIs), selective norepinephrine reuptake inhibitors (SNRIs), and complementary therapies have also been helpful. Disease-modifying therapy includes hydroxyurea, which raises fetal hemoglobin levels and reduces incidence of vaso-occlusive crises and episodes of acute chest syndrome. Hydroxyurea has been found to cause birth defects in animals, but has not been found to increase risk of birth defects in humans.

 Nonetheless, hydroxyurea has generally been avoided during pregnancy. L-glutamine has recently been approved for prevention of vaso-occlusive crises, but there is little experience with this medication.

 Other disease-modifying therapies are currently in development, but have not been approved for use.

Cures of SCD have been accomplished with hematopoietic stem cell transplantation (HSCT) and now with gene therapy.

 For HSCT, the donor may be related (e.g. a sibling) or unrelated. Related donors can be human leukocyte antigen (HLA) histocompatibility-matched (typically at
	8/8 HLA loci) or haploidentical (matched at half of HLA loci). Originally only matched donors were considered, but now successful transplants have occurred with haploidentical donors as well. In preparation for HSCT, recipients receive chemotherapy or radiation. HCST offers a cure, but can result in death, graft rejection, graft versus host disease, and sterility.

 After HSCT, a high proportion of young women do become amenorrheic and are presumed infertile.  Gene therapy for SCD

 is still experimental but there are three clinical trials underway.  Subjects receive their own genetically modified hematopoietic stem cells. In preparation, however, they still require gonadotoxic chemotherapy with the same potential risks to fertility as those with HSCT.  

 Menstruation and contraception in young women with SCD

Typical case: A 16-year-old gravida 0 with SCD is referred by her pediatric hematologist because of heavy, painful periods since she started having periods 1 year ago. She reports that the menstrual pain is distinct from the pain of vaso-occlusive crises. Her disease is otherwise adequately managed. She has no history of stroke or venous thromboembolism (VTE). Her Hb is 9 g/dL. A transabdominal ultrasound of the pelvis was normal.

Delay in menarche is not uncommon in girls with SCD. Several studies have reported on delayed menarche among girls with the disease.

 A longitudinal cohort study of girls from infancy to young adulthood in Jamaica found that compared to controls, girls with SC genotype had a delay in mean age at menarche of 0.5 years and girls with SS genotype had a delay in mean age at menarche of 2.4.

 This patient’s ability to distinguish menstrual pain from the pain with vaso-occlusive crises is also not uncommon. Women with SCD do report distinct differences in the pain of menstruation compared to the pain with vaso-occlusive crises.

Management of primary dysmenorrhea includes hormonal contraceptives and NSAIDs. In SCD, NSAIDs may increase vascular, bleeding, and renal risks, which may be compounded in patients with end-organ co-morbidities. For patients with SCD without a contraindication, however, NSAIDs are acceptable.

 Hormonal therapy can reduce blood loss and pain with periods (a potential benefit in SCD), but combined hormonal contraceptives increase risk of VTE and stroke

 (a potential hazard in SCD). Depot medroxyprogesterone acetate (DMPA) injections have also been shown to increase risk of VTE,

 but other progestin-only contraceptives do not.

 In women with SCD, a systematic review of four studies showed no increased risk of VTE among those who used hormonal contraceptives, but there were only 118 total subjects.

 Obviously, no combined hormonal contraceptives should be prescribed to a young woman with a history of stroke or VTE. Common sense further dictates progestin-only contraceptives be prescribed to a woman with SCD over combined hormonal contraceptives whenever possible.

 of a young woman referred for HSCT describes a scenario that has become more frequent. This patient was a 19-year-old young gravida 0 with SS genotype. She had a history of multiple episodes of vaso-occlusive pain crises and acute chest syndrome. Fortunately, her sister was an HLA match. She was referred to a reproductive endocrinologist who counseled her and her family regarding the options for cryopreservation of:

embryos (created with donor sperm), which allows for preimplantation genetic testing (PGT)

ovarian tissue – still experimental, but the only option for pre-pubescent girls.

A low-dose ovarian stimulation protocol was used. Enoxaparin 30 mg subcutaneous every 12 hours was administered during stimulation and held 24 hours before egg retrieval. Eight mature oocytes were vitrified (rapidly cooled to prevent formation of crystals). No reports exist about whether this patient has pursued a pregnancy successfully, but thousands of women without SCD have had successful pregnancies after oocyte preservation.

 Also, while ovarian tissue preservation is considered experimental, more than 87 subsequent pregnancies have been reported, including two in women with SCD (one using her own ovarian tissue and another using her sister’s).

A typical case: A 28-year-old gravida 0 with SCD with SS genotype is referred by her hematologist because she will be married soon and is planning a pregnancy. She is currently taking hydroxyurea. Her partner does not think he has sickle trait or beta thalassemia minor, but he is not certain. 

This patient’s partner should be referred for testing. If he has abnormal Hb, the couple should be referred for genetic counseling and they should be aware of the option of PGT with in vitro fertilization. A type and screen should be reviewed or obtained. If antibodies are present that are known to cause hemolytic disease, the partner should be tested for the corresponding antigen(s). The patient should be counseled regarding the maternal and fetal risks of SCD in pregnancy. Although the risk of maternal mortality is increased, the absolute risk of maternal mortality is about 1%,

 which does not discourage most women and their families from pursuing a pregnancy. The patient’s medications should be reviewed and before a prenatal vitamin is prescribed, her ferritin should be checked. If the level is elevated, which is very likely if the patient has received transfusions, she should receive a prenatal vitamin WITHOUT iron (such as prenatal gummies). In addition, the American College of Obstetricians and Gynecologists recommends 4 mg of folate per day for women with SCD.

 With respect to hydroxyurea, the patient should have a consultation with a maternal-fetal medicine specialist. After consultation with that specialist and a hematologist regarding risks and benefits, some patients may elect to continue hydroxyurea during pregnancy or at least until conception, stop for the duration of pregnancy, or stop it temporarily and restart it after the first trimester.  

A typical case: A 32-year-old gravida 3 para 1011 (one term pregnancy and one miscarriage) with SCD (SS genotype) at
	36 weeks’ gestation has worsening fetal growth restriction (FGR), which is now at the fourth percentile. Amniotic fluid volume is low normal and the umbilical artery Doppler S/D ratio is normal. The patient has been hospitalized almost monthly since conception for vaso-occlusive crises and was recently discharged from the medical intensive care unit after an admission for acute chest syndrome. She has received multiple transfusions in the past. Her antibody screen was positive for anti-C antibodies in this pregnancy, but her titer never exceeded 4. She took hydroxyurea in the past but stopped prior to her first pregnancy. The patient’s family history is significant for a sister who also had SCD with SS genotype and died in the second trimester of her first pregnancy. Her partner’s sickle cell status is unknown. The patient’s current medications include prenatal gummies, low-dose aspirin for preeclampsia prevention, and oxycodone for pain. Her only complaint is near constant, severe pain in her bones. Her vital signs are normal, and her exam is unremarkable. Her Hb is 7.6 g/dL, hemoglobin 22%, creatinine 0.6, and lactate dehydrogenase is normal.  

Once pregnancy is established, a patient with SCD should be cared for with the help of specialists in maternal-fetal medicine and hematology who have expertise in SCD. In early pregnancy, this patient’s partner should have been offered testing. If he had abnormal Hb, the couple should have been referred for genetic counseling and made aware of the options for prenatal diagnosis. He also could have been tested for the C red cell antigen. Anti-C antibodies are a rare but documented cause of hemolytic disease of the newborn.

 If the partner were negative, the fetus would not have been at any risk for alloimmunization. 

Because of the increased risk of preeclampsia, low-dose aspirin was started at 12 weeks’ gestation. Because of the increased risk of infection, monthly urine cultures were ordered. The patient’s hemoglobin was monitored. A Hb of 8 g/dL was targeted and a Hb of
	> 7 g/dL was maintained for most of the pregnancy. There is no good-quality evidence that a strategy of prophylactic blood transfusion does or does not benefit the mother or fetus when compared to selective transfusion.

 Whatever the transfusion strategy, the benefits of transfusion need to be balanced against the risks of alloimmunization and delayed hemolytic transfusion reactions, which can be life-threatening.

Because of the high risk of FGR, fetal growth was monitored with serial ultrasounds.

 Our practice is to start at 24 to 28 weeks’ gestation. Due to the increased risk of stillbirth, a plan for fetal surveillance was also in place. Our practice is to start antepartum testing at 32 weeks’ gestation. If all is going well, we plan to deliver women with sickle cell anemia (SS and S-beta thal

 genotypes) at 37 weeks’ gestation and women with other genotypes at 39 weeks. For the patient on opioid medication, the nursery needs to be prepared for neonatal withdrawal syndrome. During hospitalization, there should be a plan for VTE prophylaxis. It is our practice to prescribe prophylactic doses of low-molecular-weight heparin for 6 weeks postpartum.  

This patient was transfused with 2 units of RBCs prior to induction of labor. She delivered a 2400-g infant vaginally. Postpartum, she received thromboprophylaxis with enoxaparin. She planned to use a long-acting reversible contraceptive and restart hydroxyurea postpartum.

For a variety of reasons, women with SCD may be at risk of early menopause or even premature ovarian failure, but there are essentially no data about menopause in these patients.

 With a life expectancy of 50 years, however, women with SCD will need the help of gynecologists to manage menopausal symptoms. 

Increasing therapeutic options for women with SCD and a longer life span are changing how ob/gyns care for women with SCD. For more information please visit the website for the Foundation for Women and Girls with Blood Disorders at 

Hassell KL. Population estimates of sickle cell disease in the U.S. 

Michlitsch J, Azimi M, Hoppe C, et al. Newborn screening for hemoglobinopathies in California. 

Chaturvedi S, DeBaun MR. Evolution of sickle cell disease from a life-threatening disease of children to a chronic disease of adults: The last 40 years. 

Yu CK, Stasiowska E, Stephens A, Awogbade M, Davies A. Outcome of pregnancy in sickle cell disease patients attending a combined obstetric and haematology clinic. 

Al Jama FE, Gasem T, Burshaid S, Rahman J, Al Suleiman SA, Rahman MS. Pregnancy outcome in patients with homozygous sickle cell disease in a university hospital, Eastern Saudi Arabia. 

Oteng-Ntim E, Meeks D, Seed PT, et al. Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. 

Thame M, Lewis J, Trotman H, Hambleton I, Serjeant G. The mechanisms of low birth weight in infants of mothers with homozygous sickle cell disease. 

Kauf  TL, Coates T, Huazhi L, Mody-Patel N, Hartzema AG. The cost of health care for children and adults with sickle cell disease. 

Ngo C, Kayem G, Habibi A, et al. Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions. 

Telen MJ. Beyond hydroxyurea: new and old drugs in the pipeline for sickle cell disease. 

Vichinsky E, Hurst D, Earles A, Kleman K, Lubin B. Newborn screening for sickle cell disease: effect on mortality. 

Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. 

Ghafuri DL, Stimpson SJ, Day ME, James A, DeBaun MR, Sharma D. Fertility challenges for women with sickle cell disease. Expert Rev 

Dovey S, Krishnamurti L, Sanfilippo J, et al. Oocyte cryopreservation in a patient with sickle cell disease prior to hematopoietic stem cell transplantation: first report. 

Demirci S, Uchida N, Tisdale JF. Gene therapy for sickle cell disease: An update. 

Three cases illustrate the unique challenges ob/gyns face while caring for SCD patients at various life stages.

Vol 64 No 07

Ultrasound errors to avoid: How important is the report?

Considerations when discussing Essure removal

Is it possible to make OB coding less complicated?

Reproductive issues in sickle cell disease

Building bridges

Will vacuum delivery go the way of vaginal breech delivery?

Making chronic pelvic pain a little less painful

Ensuring medications are more affordable without stifling innovation (Part 2)

Which factors predict likelihood of mesh revision?

Study supports noninvasive testing for Down syndrome in twins

How likely are adolescents to fill antibiotic prescriptions for STIs?